Objective:To classify the etiology of secondary hemophagocytic syndrome (sHLH) and explore its clinical, laboratory and therapeutic characteristics in order to deepen the understanding of the disease.Method:A retrospective observational study was conducted on sHLH patients who were treated at Peking University People's Hospital from January 2016 to December 2021. Patients under the age of 18 and those with missing clinical data were excluded. The distribution of departments visited and etiologies of sHLH were analyzed. Baseline data, clinical characteristics, complications, laboratory data, treatment, and in-hospital outcomes of sHLH were collected. The sHLH patients were then divided into 3 groups including malignancy group, macrophage activation syndrome (MAS) group and other etiologies (mainly infection) group. Intergroup comparisons were performed using chi-square tests, analysis of variance, Mann-Whitney tests, and other statistical methods.Results:A total 169 patients were enrolled, among these patients, 27.8% were malignancy-related HLH, 47.9% were MAS, and 24.3% were other etiologies related HLH. Statistical analysis revealed that the clinical characteristics of other etiological group was highly consistent with the malignancy group, including more and severer peripheral blood cell reduction, higher sCD25 levels, more Epstein-Barr virus infection, and the prognosis was similar, both were with more than 50% in-hospital mortality. And the incidence of hemophagocytosis was highest in other etiological groups (65.9%). In contrast, MAS group was with an obviously lower mortality of 17.3% ( P<0.05). Meanwhile, treatments including methylprednisolone pulse, cyclosporine A and interleukin-2 were used frequently in MAS group. Conclusion:Malignancy related HLH and other etiologies related HLH exhibit more similar clinical characteristics and prognosis, while the MAS group, has a milder overall condition and better prognosis.

Objective:To explore the application of typical tasks-based mind mapping in nursing teaching for children with autism spectrum disorder (ASD).Methods:A total of 102 nursing students who were involved in the nursing of children with ASD in Hunan Children's Hospital were selected and divided into control group and observation group according to the teaching methods. Fifty-one students in the control group were provided with conventional teaching, while 51 students in the observation group were provided with typical tasks-based mind mapping teaching. The students in the two groups were assessed for performance, self-directed learning ability score, and overall literacy at completion of the nursing course. SPSS 22.0 was used for the t test. Results:The scores of theoretical examination[(92.34±4.07) vs. (89.92±3.61)], nursing note writing[(91.07±3.84) vs. (88.60±3.59)], and operational examination[(90.47±2.98) vs. (88.52±2.73)] were significantly higher among students in the observation group than among those in the control group ( P<0.05); after the internship, students in the two groups had significantly increased scores in interpersonal relationships, learning awareness, learning strategies, learning behaviors, and learning evaluation, and the observation group had better performance than the control group in the above indices ( P<0.05); after the internship, students in the two groups had significantly increased scores in problem solving, interpersonal communication, critical thinking, and self-leadership, and the observation group had better performance than the control group in the above indices ( P<0.05). Conclusion:The application of typical tasks-based mind mapping in nursing teaching for children with ASD can improve nursing students' academic performance, enhance their self-directed learning, and improve their overall literacy.

  Objective  To study the demographic and clinical characteristics, correlation of genotype and phenotype and treatment of Blau syndrome to facilitate early diagnosis and timely treatment of Blau syndrome.  Methods  Seventy-two patients with Blau syndrome from 11 centers from May 2006 to April 2022 were retrospectively analyzed, and their general information, clinical data, laboratory examination and treatment medication were collected.  Results  The distribution of patients with Blau syndrome was uniform in geographical north and south of China, and there was no obvious gender bias. The mean age of onset was (14.30±12.81) months, and the age of diagnosis was (55.18±36.22) months. 35% of patients with Blau syndrome happened before 1 year old, and all patients developed before 5 years old. 87.50% (63/72) had granulomatous arthritis, 65.28% (47/72) had rash, 36.11% (26/72) had ocular involvement, 27.78% (20/72) had fever, and 15.28% (11/72) had pulmonary involvement. Arthritic manifestations of Blau syndrome were most at risk, followed by rash, ocular involvement, and fever.The first 25 months of the disease, the risk of developing a rash was the greatest. The risk of developing arthritis was the greatest between 25 months and 84 months. The main mutations were p.R334Q and p.R334W, and patients with p.R334Q mutation had relatively high incidence of fever (35.71%[5/14] vs. 14.29%[1/7], P=0.43) and ocular involvement (42.86%[6/14]vs. 28.57%[2/7], P=0.51). There was a relatively high incidence of rash (85.71%[6/7] vs. 64.29%[9/14], P=0.59) in patients with the p.R334W mutation. Forty-five patients(62.50%)were treated with a combina-tion of glucocorticoid and methotrexate. Twenty-two patients were treated with tumor necrosis factor antagonist in addition to glucocorticoid and methotrexate.  Conclusions  The risk of different clinical manifestations of Blau syndrome from high to low was arthritis, followed by rash, ocular involvement and fever. The main treatment was glucocorticoid combined with methotrexate, to which biological agents could be added.

Objective: To explore the roles of CD34, VEGF and endothelin-1 in hepatic sinusoid capillarization and the intrahepatic metastasis of hepatocellular carcinoma. Methods: Data of 10 cases of hepatocellular carcinoma without intrahepatic metastasis were compared with those 10 cases of hepatocellular carcinoma with intrahepatic metastasis . Liver cancer tissues and paracancerous tissues were collected, hepatic sinusoidal capillarization was detected by immunohistochemistry, mRNA expression and protein expression of CD34, VEGF and endothelin-1 genes were detected by QPCR, Western blot, and the relationship between gene expression and metastasis of HCC was observed. Results: Hepatic sinusoid capillarization was observed in carcinoma tissues, not in the adjacent tissues, whereas mRNAs and proteins of CD34, VEGF and endothelin-1 were higher in liver cancer tissues than that in the adjacent tissuess. In addition, both mRNA and protein expression levels of these genes were significantly higher in the intrahepatic metastatic liver cancers than those without metastasis (P<0.05). Conclusion CD34, VEGF and endothelin - 1 may play important roles in intrahepatic metastasis of liver cancer.

Objective To investigate the clinical characteristics and prognosis of patients with acute aortic dissection (AAD) and hypertension,and explore other related prognostic factors in AAD.Methods The present study enrolled consecutive patients diagnosed with AAD who were admitted to Peking University People's Hospital between January 2000 to December 2015.Patients diagnosed with AAD by CT angiography,aortography or magnetic resonance imaging within 14 days of onset were included.Patients with infectious diseases,haematological diseases,malignancies,autoimmune diseases and patients without clearly clinical diagnosis or incomplete data were excluded.The patients were initially divided into two groups based on their history of hypertension,and their clinical characteristics were compared and analyzed.We further divided AAD patients into survival group and death group according to their in-patient outcomes,and factors related to their prognoses were analyzed.Logistic regression analysis was applied to analyze the independent risk factors related to hospital death in AAD patients with P＜0.05 as the significant value.Results The hypertensive group contained 237/346 cases included (68.45％),patients in this group were generally older than their non-hypertensive counterparts,accompanied by increased prevalence of comorbidities (coronary heart diseases or diabetes) and a statistical significant elevated admission blood pressures (systolic and diastolic,P＜0.05).No significant difference were found between the groups in terms of white blood cell and platelet count,D-dimer,neutrophil to lymphocyte ratio(NLR),fibrinogen,serum creatinine and serum lipid profiles (P＞0.05).Hypertensive patients were less likely to receive surgical treatment compared with those without hypertension(P＜0.05),with increased risk of in-hospital mortality (P＞0.05).Further logistic regression analysis revealed the presence of hypertension did not independently predict in-hospital mortality of AAD patients.Factors such as age,Stanford classification of the AAD,NLR and platelet counts were found to have independent predictive values for in-hospital mortality (P＜0.05).Conclusion AAD patients with hypertension are generally older,have more comorbidities such as coronary heart diseases and diabetes.The presence of hypertension itself is not directly associated with in-hospital mortality in AAD patients,while the Stanford classification,age,NLR and platelet counts are independent risk predictors.

Objective: To investigate the effect of siRNA silencing α1 antitrypsin (α1-AT) gene on the biological behavior of rheumatoid arthritis fibroblast-like synoviocytes (RA-SFs). Methods: Primary culture of knee synovial tissue from 5 patients with rheumatoid arthritis (RA) was performed. The artificially synthesized silencing α1-AT siRNA specifically inhibits the expression of α1-AT in RA-SFs. After 24 and 36 hours of transient transfection, the inhibition efficiency was detected by Quantitative reverse transcription polymerase chain reaction (RT-qPCR), and the expression of related genes after α1-AT gene silencing was detected.Furthermore, ethyl thiazolyl tetrazolium (MTT) assay, Trans-well chamber, cell scratch and enzyme-linked immunosorbent assay (ELISA) were used to detect the effects of interfering α1-AT expression on cell proliferation, invasion and migration, and secretion of interleukin (IL)-17, Tumor necrosis factor (TNF)-α, IL-1α, IL-1β and other related inflammatory factors. At the same time, when the pathway inhibitor (ERK inhibitor, signal transducer and activator of transcription 3 (STAT3) inhibitor, NF-κB inhibitor) stimulated cells, the effect on α1-AT was changed. One-way analysis of variance was used for comparison between the two groups; further pairwise comparison using LSD-t test; The count data was checked by χ² test. Results: Compared with the negative control group, the siRNA α1-AT group was transfected for 24 hours, the α1-AT mRNA level was significantly inhibited (P<0.05), and the proliferation rate of RA-SFs was not affected (P>0.05), and the invasion and migration ability of cells decreased significantly (P<0.05). The secretion of IL-17 and IL-1β was slightly decreased (P>0.05), while TNF-α and IL-1α were not affected (P>0.05). In addition, the expression of α1-AT downstream genes Matrix metallop roteinases (MMP)-2 169, Hu-Bax1, MMP-9, Hu Bax and Hu Bcl-xl were significantly decreased (P<0.05). Moreover, the signaling pathway inhibitors ERK inhibitor (PD98059) and NF-κB inhibitor (PDTC) had significant effects on α1-AT (P<0.05). Conclusion The α1-AT gene silencing has potential effect on the biological behavior of RA SFs. Further study of this mechanism is helpful to provide evidence for the treatment of RA.

Objective To compare and analyze the dosimetric discrepancy of combind intracavitary/interstitial brachytherapy using three different kinds of optimization method in locally advanced cervical cancer.Methods Totally 20 cases of locally advanced cervical cancer were selected and divided into three groups according to different optimization method which include manual optimization group (MO) based on graphical optimization,inverse planning simulated annealing (IPSA 1)based on simulated annealing optimization algorithm,IPSA 2 based on IPSA 1 with limitation on maximum dose of target.The dose volume histogram parameters of the targets (V200,V150,V100,D100,D90,HI) and the OARs(D0.1 cm3,D1 cm3 and D2 cm3) were analyzed.Results For CTV,compared with MO,there was no significantly statistical difference in D100between IPSA 1 and IPSA 2(P ＞ 0.05).However,V200,V150,V100 and HI for ISPA1 were better than for ISPA2 (t =-3.422-9.910,P ＜ 0.05).In addition,V100 and D100 in ISPA1 were better than in ISPA2 (t =7.238,5.032,P ＜0.05).For OARs,D0.1 cm3,D1 cm3 and D2 cm3 in rectum,bladder,sigmoid colon of both ISPA 1 and ISPA 2 were dramatically lower than those of MO (t =2.235 5.819,P ＜ 0.05),without significantly statistical difference found between ISPA1 and ISPA2.Conclusions For combined intracavitary/interstitial brachytherapy in locally advanced cervical cancer,all treatment plans based on three different kinds of optimization methods can meet the clinical need.Moreover,inverse optimization can ensure dose coverage over target and reduce maximum dose of rectum,bladder and sigmoid colon.

Objective: To explore the effects of microRNA-34a on regulating silent information regulator 1 (SIRT1) and influence of SIRT1 on myocardial damage of rats with severe burns at early stage. Methods: (1) Twenty-four Sprague-Dawley (SD) rats were divided into sham injury (SI) group, simple burns (SB) group and SIRT1 agonist (SA) group according to the random number table (the same grouping method below), with 8 rats in each group. Rats in groups SB and SA were inflicted with 30% total body surface area full-thickness scald (hereinafter referred to as burns) on the back, and rats in group SI were sham injuried on the back. Immediately after injury, rats in groups SI and SB were intraperitoneally injected with normal saline of 50 mL/kg, and rats in group SA were intraperitoneally injected with normal saline of 50 mL/kg and 1 mg/mL resveratrol of 50 mg/kg. At 6 h post injury, abdominal aortic blood was collected to make serum and myocardial tissue of rats was collected. (2) Myocardial cells of twelve neonatal SD rats were collected and divided into microRNA-34a mimic control (MMC) group, microRNA-34a mimic (MM) group, microRNA-34a inhibitor control (MIC) group, and microRNA-34a inhibitor (MI) group, which were respectively transfected with gene sequences of mimic control, mimic, inhibitor control, and inhibitor of microRNA-34a. The microRNA-34a expression level and protein expression level of SIRT1 in myocardial cells were respectively detected by real-time fluorescence quantitative reverse transcription polymerase chain reaction (RT-PCR) and Western blotting. Another batch of myocardial cells were divided into microRNA-34a inhibitor control+ burn serum (MCB) group, microRNA-34a inhibitor+ burn serum (MB) group, and microRNA-34a inhibitor+ burn serum + EX527 (MBE) group. Myocardial cells in group MCB were transfected with gene sequence of inhibitor control, and myocardial cells in the later groups were transfected with gene sequence of inhibitor of microRNA-34a. After transfection of 48 h, myocardial cells in group MBE were cultured in Dulbecco′s modified Eagle′s medium (DMEM) solution for 6 hours, with serum in group SB of volume fraction of 10% and final amount-of-substance concentration of 1 mol/L, and myocardial cells in the other 2 groups were cultured in DMEM solution with serum from rats of group SB of volume fraction of 10%. The protein expression levels of myocardial cells of SIRT1, cleaved-caspase-3, and Bax were detected by Western blotting. (3) Myocardial tissue from (1) was collected to detect expression levels of microRNA-34a and mRNA of SIRT1 in groups SI and SB by real-time fluorescence quantitative RT-PCR. Morphology of myocardial tissue of rats in groups SI, SB, and SA was observed with biological image navigator. The mRNA expression levels of interleukin 1β (IL-1β) and tumor necrosis factor (TNF-α) of rats in groups SI, SB, and SA were detected by real-time fluorescence quantitative RT-PCR. The expression levels of cleaved-caspase-3, and Bax of myocardial tissue of rats in groups SI, SB, and SA were detected by Western blotting. Data were processed with one-way analysis of variance and least-significant difference test. Results: (1) After transfection of 48 h, the expression level of microRNA-34a of myocardial cells in group MM was 4.67±0.92, significantly higher than 1.03±0.04 in group MMC (P<0.01); the protein expression level of SIRT1 of myocardial cells in group MM was 0.35±0.06, significantly lower than 1.12±0.11 in group MMC (P<0.01). After transfection of 48 h, the expression level of microRNA-34a of myocardial cells in group MI was 0.26±0.07, significantly lower than 1.33±0.07 in group MIC (P<0.01); the protein expression level of SIRT1 of myocardial cells in group MIC was 1.12±0.16, significantly lower than 1.74±0.34 in group MI (P<0.01). At 6 h after culture, compared with those in group MCB, the SIRT1 protein expression level of myocardial cells in group MB was significantly increased (P<0.05), while cleaved-caspase-3 and Bax protein expression levels of myocardial cells in group MB were significantly decreased (P<0.05). Compared with those in group MB, the SIRT1 protein expression level of myocardial cells in group MBE was with no significantly statistical difference (P>0.05), and cleaved-caspase-3 and Bax protein expression levels were significantly increased (P<0.05). (2) At 6 h post injury, compared with that in group SI, the microRNA-34a expression level of myocardial tissue in group SB was significantly increased (P<0.01), and the mRNA expression level of SIRT1 of myocardial tissue in group SB was significantly decreased (P<0.01). At 6 h post injury, myocardial cells in group SI arranged neatly with normal nucleus and no inflammatory cells infiltration; myocardial cells in group SB arranged disorderly, with no abnormal nucleus, and obvious inflammatory cells infiltration; myocardial cells in group SA arranged neatly, with normal nucleus and little inflammatory cells infiltration. At 6 h post injury, compared with those in group SB, the mRNA expression levels of IL-1β and TNF-α, and the protein expression levels of cleaved-caspase-3 and Bax of myocardial tissue in groups SI and SA were significantly decreased (P<0.01). Conclusions The microRNA-34a expression level of myocardial tissue of rats with severe burns at early stage increases, which decreases the expression level of SIRT1, and increases the expression levels of IL-1β, TNF-α, cleaved-caspase-3 and Bax, leading to obvious myocardial damage. Activation of SIRT1 can alleviate myocardial damage of rats with severe burns at early stage through decreasing expression levels of IL-1β, TNF-α, cleaved-caspase-3, and Bax.

Objective: To observe the effects of music electro-acupuncture and pulsed electro-acupuncture on locomotor avtivity and hippocampal astrocytes in fast-aging (SAMP8) mice, and to compare the anti-dementia mechanism.Methods: Thirty 8-month-old male SAMP8 mice were randomly divided into model group (group M), music electroacupuncture group (group MA), pulsed electro-acupuncture group (group EA) (n=10), with homologous normal aging SAMR1 mice as control group (group C) (n=10) . Acupuncture stimulation was applied to"Baihui" (GV20) 、"Yintang" (GV29) and"Renzhong" (DU26) for 20 min per day for 15 days. The Morris water maze was used to assess learningmemorize ability. Immunohistochemical DAB staining was used to observe GFAP exptession in hippocampus. Detect GFAP protein levels by Western blot in hippocampus. Results: The Morris water maze test showed: Compared with group C, the escape latency were increased (P ＜ 0.01), swimming distance were increased in space exploration experiment in group M (P ＜ 0.01) . Compared with group M, the escape latency were reduced in group MA and EA (P ＜ 0.01, P ＜ 0.05) and the swimming distance were shortened (all P ＜ 0.05) . The immunohistochemical DAB staining showed: Compared with group C, the average optical density of GFAP in the hippocampal CA1 area of group M increased significantly (P ＜0.01); the optical density of GFAP in CA1 area of hippocampus in both groups MA and EA was significantly decreased (all P ＜ 0.01), while the optical density of GFAP in MA group was lower than that of EA group (P ＜ 0.05) . Western blot test showed: Compared with group C, the expression of GFAP protein obviously increased (P ＜ 0.01) . Compared with group M, the expression of GFAP protein obviously decreased and had a downregulation tendency in group MA and EA (all P ＜ 0.05), while the expression of GFAP protein decreased in MA group compare with EA group (P ＜ 0.05) .Conclusion: Both music electro-acupuncture and pulsed electro-acupuncture can improve the learning and memory abilities of SAMP8 mice and decrease the expression of GFAP, which may be related to its mechanism of reducing neuroinflammatory reaction, improving apoptosis and eventually protecting neurons, and music electro-acupuncture has a better tendency than pulsed electro-acupuncture.

Objective To investigate the clinical characteristics and associated prognostic factors of the acute aortic syndrome. Methods The clinical data of 391 patients with acute aortic syndrome (AAS) admitted to Beijing University People's Hospital from January 2000 to December 2015 were analyzed. Results In 391 patients with AAS, the average age was (52.7 ±13.3) with a male/female ratio of 4.3:1, and 73.4％ patients had hypertension. The most common clinical manifestation of patients with AAS was pain accounting for more than 90.0％, and the nature of pain was expansible and/or transitive pain. The level of D-dimer was elevated in 91.1％ of patients with AAS (ELISA), and significantly higher in type A patients than type B patients. Ultrasound/echocardiography was used to diagnose AAS with a sensitivity of 88.4％, of which the sensitivity of type A patients was 99.1％; the mortality of type A patients was significantly higher than type B patients (34.3％ vs. 0.9％, P <0.01). The mortality was declined obviously (11.7％ vs. 28.0％, P <0.01) when patients were treated with stent. Compared with the in-hospital surviving group, the in-hospital mortality group had decreased platelet counts and FIB, higher level of D-dimer, FDP and NLR (neutrophil to lymphocyte ratio). Conclusions Transitive and(or) expansible pains were the characteristic clinical manifestations of acute aortic syndrome,which usually happened suddenly or tearing;D - dimer and ultrasound were valueble and simple methods in AAS patients; Compared with In-hospital surviving group,the In-hospital mortality group had decreased platelet counts and FIB,higher level of D-dimer, FDP and NLR.The mortality of type A was significantly higher than type B,operation can lower the mortality of AAS patients obviously.